6. congenital heart disease

Congenital Heart Disease

For congenital heart disease (CHD), we mainly do treatment for adolescent or adult patients with the CHD.

However, when a child patient has a disease which is eventually same as adult one such as cardiomyopathy or heart failure, we may be able to help him/her.

In any case, we discuss treatment and strategy with pediatric cardiologists or pediatric cardiovascular surgeons, and we combine the different but important/useful viewpoints together toward the best treatment and care.

We would like to discuss the adult congenital heart disease which we do treatment.

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Aortic valve disease （Aortic regurgitation or stenosis due to Bicuspid Aortic valve）

Bisucpid Aortic Valve (Healthy Aortic Valve usually has 3 cuspis, but some people have 2) occurs every 100 birth. The valve tend to have stenosis (narrow valve) or regurgitation (leaking valve), and may develop infectious endocarditis (IE, bacteria grow in/around the valve and heart, and cause critical infection).

  Indication of surgery is similar to usual Aortic stenosis (chest pain, syncope, congestive heart failure or valve area becomes less than 0.75cm2) or Aortic regurgitation (symptom, severe regugitation, LV dilatation or disfunction).  In addition, patients with bisucpid aortic valve have structural weakness in aortic wall, and when their ascending aorta dilates, it may have to be repaired or replaced. The strategy may prevent future presence or rupture of the aneurysm. Aorta of the patient with bicuspid aortic valve may have to be treated in  a way similar to connective tissure disease such as Marfan syndrome or Ehler-danros syndrome.

Bicuspid valve with aortic regurgitation is often repairable.  When the aortic cusps are intact, young patients especially on their 10s or 20s can befefit a lot from the repair surgery of the aortic valve. Patients with the age cannot have a happy school life with mechanical valve and warfarin, and they cannot have intact tissue valve (bioprosthesis) more than 10 years; valve repair should be the choice for them. Here we present a case who had aortic valve repair on his teens, and doing well for 10 years. (Aortic valve repair case)

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Atrial Septal Defect (ASD)

Atrial septal defect (ASD) is one of the most common congenital heart diseases, where the patient have no strong symptoms for long time and the disease often undiagnosed in screening check at shool. Sometimes the disease is diagnosed when the patient is on his/her 50s or 60s.

When a patient with ASD becomes over 40 years old, his/her QOL (quality of life) is impaired by congestive heart failure, atrial fibrillation, or dyspnea on effort. Only half of ASD patients survive and reach their 50s, and they have mortality of 6%/year thereafter. That is a reason ASD patients even over 60 years old often have an indication of surgery.

When a left-to-right shunt via ASD is too much (e.g., amount of blood flow in the lung is 2 times as much as the flow of whole body), or when there is a right-to-left shunt (cerebral infarction can occur), surgery is indicated.

Recently ASD closure by catheter is being tried. But the promising method has limitations depending on ASD type and location etc. It may cause incomplete closure and long-term results remain unknown.

Sugery has a weakpoint of making scar on the skin, but surgery has a point in that its safe, sure and can handle various secondary problems such as tricuspid regurgitation, mitral regurgitation, right atrial dilatation, and atrial fibrillation etc. Tricuspid valve and mitral valve can be repaired. Atrial fibrillation can be fixed by Cox-Maze procedure.  We kept woking on the atrial fibrillation for years (e.g. more than 10 years), and depending on the atirial condition we do atrial (volume) reduction Maze procedure which has higher defibrillation rate and better postoperative atrial function.

In all, approach which considers the patient's conditions carefully is important.  So far surgery of ASD yields 100% success rate. We see more patients who have other concomitant diseases and thus we believe that we should be more careful than before for the patients.

We treat ASD which accomnapnies Ebstein's disease in case by case fashion. After considering RV/LV function and size, direction and amount of shunt at the ASD, degree of pulmonary hypertension, we do ASD repair as well as repair/replacement of tricuspid valve and anti-arrhythmia surgery (such as Cox-Maze procedure).  In the treatment of adult congenital heart disease, we collaborate with congenital heart surgeon.

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Ventricular Septal Defect (VSD)

This is a disease with a hole (holes) in the interventicular septum. There are 4 types in the disease depending on the location of the hole: membraneous septum, infundibular septum, muscular septum, inflow septum.

When the hole is midium size or larger, surgery can be considered.  Also, when the shunt via the hole is too big, or when the LV is overloaded too much, or when there is a reversible pulmonary hypertension, surgery can be indicated.

When the defect is medium size or smaller, it can heal of itself and thus it does not be a surgical candidate. When the small defect does not close by itself even after adolescent, surgery may be perfomed.

When the location of VSD is in proximal septum, aortic cusp can be prolapsed and aortic regurgitation may develp; aortic valve repair or replacement can be indicated.

When the VSD is small, infectious endocarditis (IE) may develop and we should be careful about it.  If IE develops, even when it is healed by antibiotics, there is a high chance of recurrence of the IE as shown by EBM (evidence based medicine). In fact, some patients agreed with surgery after the recurrence of IE.

Surgery of VSD is safe, but if concomitant surgery becomes necessary, the surgery  may be more risky. Therefore, proper timing of the surgery is important; it should not postponed too much.

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Patent Ductus Arteriosus (PDA)

PDA is a disease where a vessel which worked during a fetus time remains patent late after birth.

When PDA is large, heart failure or pulmonary hypertension can develop, and thus surgery is indicated. When the PDA is relatively small, it may cause IR and usually PDA is surgically closed to avoid the problem.

Treatment of PDA includes catheter intervention which employs a coil mounted on the tip of the catheter and plug the PDA from lumen. Although the method is making progress, surgery is also improving such as small incision surgery, clipping, and thoracoscopic surgery.

PDA is in its nature an organ which degenerates as time passes, and becomes more fragile as the patient becomes older. When the patient reaches 30s, the PDA becomes calcified,and then the patient becomes over 50 the PDA tissue is further more fragile. As a result, simple ligation is no longer safe for the patient of the age, and instead, closure of the PDA orifice via pulmonary artery under cardiopulmonary bypass and hypothermia is recommended. We do the surgery by milder hypothermia on cardiopulmonary bypass, so that the surgery is less invasive. (Case: Adult PDA) 

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Pulmonary Stenosis (PS)

In PS, pulmonary valve orifice is narrowed. PS has 3 types depending on the location of the narrowing: valvular, supra-valvular, sub-valvular. Supravalvular or subvalvular narrowing is often accompanied by other cardiac diseases.

Tight stenosis is associated by sudden death and early treatment is necessary. Moderate stenosis or less is accompanied by mild stenosis and surgery is done after child age. Some patients of the group have surgery after adult age.

Treatment includes balloon valvotomy and when it does not work, surgery. In surgery, open valvotomy under cardiopulmonary bypass or valve replacement when the valve is not repairable.

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LV noncompaction

Please refer to 2. cardiomyopathy and heart faiulre. The LVNC is unknown disease for a long time, but recently more researches have been done. When medical treatment such as diuretics, ARB, or beta-blocker is not effective enough, surgery can be considered.

Relatively mildly diseased population of this disease survive until adult age and are diagnosed. Patients of milder LVNC often have heart failure or history of stroke. Patients with severe LVNC often die at their childhood.

We reported the first successful case of LV restoration surgery for LVNC (Please see Papers in English). LV restoration using patch for LVNC can prevent heart failure and LV thorombi.

By collaborating with cardiologists, pediatric cardiologists, and general physicians, we would like to do public education to deepen understanding the LVNC so that early diagnosis and early treatment can be done and when necessary surgery before the patient have major problems.

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Idiopathic Dilated Cardiomyopathy

Please see the section of 2. cardiomyopathy and heart failure. This group includes Idiopathic dilated cardiomyopathy and dilated cardiomyopathy secondary to various diseases such as myocarditis.

In children with dilated cardiomyopathy, surgery works well when LV dilatation is severe, or when LV has discrete lesion. (Periatric case of LV restoration )

For that type of diseases, we collaborate  with pediatric cardiologists or pediatric cardiac surgeons. Treatment from both viewpoints (i.e., adult and child) helps toward safety and better results.

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Sinus Vvalsalvae Aneurysm

Sinus Valsalvae is a pocket-shaped structure which is located at the root of the Aorta or at the outlet of the heart. Sinus valsalvae aneurysm develops when one or more sinus valsalva dilate; most common right sinus valsalvae aneurysm often ruptures to right ventricule and non-coronary sinus valsalvae often ruptures to right atirum.

When the rupture orifice is large, chest pair, dyspnea (difficulty of breathing), and/or heart failure develops rapidly.  In that situation, emergency surgery may be necessary.  In operation, under cardiopulmonary bypass the rupture orifice can be closed directly or by using a patch; when necessary aortic valve may be repaired. If there is concomitant diseases such as ventricular septal defect (VSD), they will be repaired at the same time. 

Risk of the operation is low (i.e. safe) when the condition before surgery is stable; under the situation even concomitant aortic valve repair/replacement can be done safely. The risk will be 1-2% on average which may be influenced by patient's age and other diseases.

When the patient develops shock before the surgery and especially when the patient is on the ventilator for quite a while, they often develop pneumonia and/or  other organ failure, and then the risk of the surgery will be higher. Early consultation can help safer operation and better outcomes.

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Coronary Fistula and/or Coronary Aneurysm

Coronay fistula is a relatively rare disease which is diagnosed in 0.2% of the patients who have coronary angiography; 30% of the patients have coronary aneurysm.  When the shunt via the coronary fistula is a lot, heart faiulre or myocardial ischemia (which mimicks angina) can develop.  Infectious endocarditis (IE) can develop. When heart failure develops, the shunt is big, or aneurysm is about to rupture, surgery is indicated.

In operation, we not only close entrance and exit of the fistula/aneurysm but also close the aneurysm itself, since the disease often develops new shunt with some other part of the heart. We try to do the surgery in Off-Pump fashion (i.e., no cardiopulmonary bypass) whenever possible.

Coronary aneurysm is known to develop after Kawasaki's disease.  In Kawasaki's disease, surgery helps to repair/stabilize the aneurysm and fix the ischemia (i.e. narrowing of the coronary arteries) which is specifically not easy to treat by catheter intervention.

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Idiopathic Hypertrophic Subaortic Stenosis (IHSS)

In IHSS, outlet of the left ventricle becomes narrows just like tunnel, and secondary mitral valve regurgitation often develops.  If the narrowing is caused by fibrous/membraneous tissue, it can be excised. If the narrowing is caused by muscles, it can be excised without bothering other structures of the ventricle.  When necessary aortic valve can be repaired or replaced.

IHSS is often accompanied by anterior shift of the mitral leaflet (SAM) which causes mitral regurgitation. When the abnormal muscles is excised in the surgery, the mitral regurgitation usually dissappears or is ameliorated automatically after the surgery. (IHSS case)

I personally had a lot of experiences of the treatment/surgery in Toronto General Hospital of University of Toronto where the specialist of adult congenital heart disease (e.g. Professor William G. Williams) especially IHSS was working and IHSS surgery was done every week. Now we do surgery for IHSS by improving the Toronto technique.  So far majority of the patients are on their 50s and 60s, which suggest the disease is in a wide range of the age.